Sigmoid mesenteric tumour as a manifestation of von Recklinghausen's disease.
The gastrointestinal tract may be involved in up to 25% of cases of von Recklinghausen's disease. The authors report such a case in which an 18-year-old girl presented with gastrointestinal symptoms secondary to a tumour in the sigmoid mesentery. There was no family history so it was presumed to be a case of spontaneous mutation. The authors discuss the protein manifestations of neurofibromatosis emphasizing the gastrointestinal presentations and the need for careful surgical management.
D000293:Adolescent; D005260:Female; D006801:Humans; D008643:Mesentery / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology; D010534:Peritoneal Neoplasms / Q000209:etiology* / Q000473:pathology; D012811:Sigmoid Neoplasms / Q000209:etiology* / Q000473:pathology